Dementia

Front 1

What are the cortical dementias?

Back 1

1. Alzheimer's Disease
2. Pick's Disease
3. Diffuse Lewy Body Dementia
4. Creutzfeldt-Jacob's Disease

Front 2

What are the subcortical dementias?

Back 2

1. Huntington's Chorea
2. Parkinson's Disease
3. Progressive Supranuclear Palsy (PSP)
4. Wilson's Disease
5. Hallerverorden-Spatz disease
6. Syndenham's Chorea
7. Subacute Sclerosing Panencephalitis (SSPE)
8. Shy Drager Syndrome
9. Olivopontocerebellar Atrophy
10. Striato-Nigral Degeneration

Front 3

What is the second most prevalent dementia?

Back 3

Multi-infacrt dementia

Front 4

Dementia is:
a) A progressive illness noted by plaques & tangles;

b) Often accompanied with cogwheel rigidity;

c) A set of symptoms which may be associated with many disorders;

d) Synonymous with declines in intellect

Back 4

c) A set of symptoms which may be associated with many disorders

Front 5

What is the essential feature of dementia?

Back 5

Memory impairment (required) AND at least one other kind of cognitive deficit (e.g., aphasia, apraxia, agnosia, executive function)

Front 6

Dementia always has an underlying organic cause.

TRUE or FALSE

Back 6

TRUE

Front 7

Estimates of the prevalence of dementia among the elderly have ranged from ____% to ___% of persons over 65 for severe demential, and ___% to ___% for those with milder dementia.

Back 7

1.3% to 6.2%

2.6% to 15.4%

Front 8

Which type of dementia affects at least half of the dementia cases?

Back 8

Alzheimer's Disease (affecting 5-6% of people above 65).

Front 9

Can a 4-year-old develop dementia?

Back 9

Yes.

"If a 4 year old developed a chronic neurologic disorder that interfered with previously acquired functions so as to significantly lower IQ or adaptive functioning, he would be considered to have both dementia and retardation."

Front 10

What is the neuroanatomical characteristic of Dementia of the Alzheimer's Type?

Back 10

A loss of cholinergic neurons in the nucleus basalis of Meynert leading to development of senile plaques and neurofibrillary tangles

Front 11

What is the age of onset for Dementia of the Alzheimer's Type?

Back 11

Between 40 and 90; most common after 65

Front 12

What are the risk factors for Dementia of the Alzheimer's Type?

Back 12

1. Female
2. Family history of Down Syndrome
3. TBI
4. History of thyroid disease

Front 13

The primary motor, somatosensory, visual, and auditory cortices are relatively spared in Dementia of the Alzheimer's Type.

TRUE or FALSE

Back 13

TRUE

Front 14

Which of the following areas is affected the most in Dementia of the Alzheimer's Type?

a. Frontal lobes
b. Medial temporal lobes
c. Basal temporal cortex

Back 14

b. Medial temporal lobes

(See pg. 873 of Blumenfeld)

Front 15

What neuroanatomical changes are seen in Dementia of the Alzheimer's Type?

Back 15

1. Cerebral atrophy
2. Neuronal loss and neurofibrillary tangles, prominent in:
a. Nucleus basalis
b. Septal nuclei
c. Nucleus of the diagonal band
3. Amyloid plaques

Front 16

In Alheimer's a dominant early feature is _________ & the next common feature is ____________, along with ____________, & ___________.

Back 16

Memory loss
Word finding difficulty
Apraxia
Visuospatial deficits

Front 17

Are individuals with Alheimer's are more likely to have problems with explicit or implicit learning?

Back 17

Explicit

Front 18

Behavioral difficulties occur early in Alzheimer's.

TRUE or FALSE

Back 18

FALSE

(Consider frontotemporal dementia if behavioral problems are present early on)

Front 19

Hallucinations are uncommon in Alzheimer's.

TRUE or FALSE

Back 19

TRUE

(Consider demential with Lewey bodies if present)

Front 20

Motor disturbances are usually present early on in Alzheimer's.

TRUE or FALSE

Back 20

FALSE

Front 21

Are neurofibriallary tangles associated with cognitive deterioration?

Back 21

There is some debate in the literature; however, most recent findings suggest that the amount of tangles is directly correlated with the degree of cognitive deterioration

Front 22

In Alheimer's, cortical atrophy, as much as 1/3 of the brain's volume as the disease progresses, occurs. Is this atrophy uniform?

Back 22

No

Most affected: Parietal tertiary areas; inferior temporal cortex; and limbic cortex

Least affected: primary sensory and motor areas

Front 23

Where are the most severe degenerative changes in Alzheimer's?

Back 23

Limbic cortex; specifically, the entorhinal cortex, which is responsible for relaying information to and from the neocortex and hippocampus. This equates to loss of the hippocampal formation.

Front 24

The strongest association between dementia and cell degeneration occurs with dysfunction in:

a) serotonergic neurons
b) norepinephrin neurons
c) cholingeric neurons
d) dopamineric neurons

Back 24

c) cholinergic neurons

Acetylcholine is clearly reduced in individuals with Alzheimer's; therefore, the treatment is focused on enhancing cholinergic function)

Front 25

What chromosomes have been found to be associated with late-onset Alzheimer's?

Back 25

Chromosomes 19 & 12

Front 26

What chromosomes have been associated with early-onset Alzheimer's?

Back 26

Chromosomes 21 & 1

Front 27

Describe Stage 1 (first 1-3 years) of Alzheimer's

Back 27

Memory - new learning deficit, mildly impaired remote memory
V/S - topographic disorientation, poor complex construction
Language - empty speech with few substantive words and paucity of ideas; anomia
Personality - indifference, occasional irritability
Motor Systems - normal
EEG - normal
CT/MRI - normal

Front 28

Describe Stage 2 (2-10 years) of Alzheimer's

Back 28

Memory - recent and remote recall more severely impaired
V/S - poor constructions, spatial disorientation; patients can not find way about or copy constructions
Language - fluent aphasia, impaired comprehension; but, relatively preserved repetition
Cognitive Skills - severely impaired
Calculation - acalculia
Personality - indifference, irritability
Motor Systems - ideomotor apraxia, restlessness, pacing
EEG - slowing of background rhythm
CT/MRI - normal or ventricular dilation and sulcal enlargement
PET/SPECT - bilateral parietal and frontal hypometabolism/hypoperfusion

Front 29

Describe Stage 3 (8-12 years) of Alzheimer's

Back 29

Memory - severely impaired
Intellectual - severely impaired
Language - reduced to echolalia, palilalia (when a word is rapidly and involuntarily repeated), or mutism
Motor Systems - limbs assume a rigid and flexed position
Sphincter control - unrinary and fecal incontinence
EEG - diffuse slowing
CT/MRI - diffuse cerebral atrophy with ventricular dilation and sulcal enlargement
PET/SPECT - bilateral parietal and frontal hypometabolism/hypoperfusion

Front 30

What are the possible causes of Alzheimer's?

Back 30

1. Genetics
2. Trace metals - aluminum salts caused similar neurofibrillary degeneration in animals
3. Immune reactions - the immune system loses its ability to recognize its own body and therefore develops antibodies which can attack the brain and cause neuronal degeneration
4. Slow viruses - which takes years to develop (like with Creutzfeldt-Jakob's disease)

Front 31

Primary progressive dementia of the Alzheimer's type usually involves a ____ deterioration.

Back 31

slow

Front 32

The first phase of Alzheimer's is _________ (noticed by the individual & sometimes those close to person) & accompanying _________ caused by forgetfulness.

Back 32

The forgetful phase

Anxiety

Front 33

In Alzheimer's the _____ phase leads to "severe deficits in memory for recent event, difficulty in _______ & _____, and ___________________ (e.g., word finding difficulty) despite generally intact vocabulary and syntax."

Back 33

Confusion

Orientation, concentration, subtle language deficits

Front 34

What does the dementia phase of Alzheimer's entail?

Back 34

Severe disorientation, abnormalities of language, perception and praxis, and behavioral problems including motor restlessness, wandering, & psychotic symptoms (paranoia).

Front 35

Pick's disease is a cortical dementia.

TRUE or FALSE

Back 35

TRUE

Front 36

Pick's disease is a rare disorder consisting of atrophy of _______ & _______ lobes from unknown causes.

Back 36

Frontal
Temporal

Front 37

At autopsy Pick's is differentiated from Alzheimer's because atrophy is confined to the _______ & _______ cortex, and _______ & ________ characteristic of Alzheimer's are not present.

Back 37

Frontal, temporal

Plaques, tangles

Front 38

Pick's patients have more memory, calculation, and visuospatial impairments than Alzheimer's patients.

TRUE or FALSE

Back 38

FALSE

Front 39

Pick's patients typically have more _________ changes than Alzheimer's patients.

Back 39

Personality

Front 40

How are verbal skills affected by Pick's disease?

Back 40

It produces aphasia, but also has a greater tendency to produce stereotyped verbal output (i.e., repeating the same story or joke over and over)

Front 41

What are Kluver-Bucy symptoms?

Back 41

Visual agnosia, emotional changes, altered sexual behavior, hypermetamorphosis and oral tendencies

Front 42

How can EEGs distinguish between Pick's and Alzheimer's patients?

Back 42

EEGs will be normal in Pick's patients during the late stages of the disease.

Front 43

Individuals with dementia due to Lewy Body Disease have hallucinations.

TRUE or FALSE

Back 43

TRUE

Front 44

Patients with Lewy Body Disease are more likely to have detailed _________ hallucinations, ___________ motor symptoms, (greater/lesser) sensitivity to antipsychotic medications, and significant ____________ in functioning.

Back 44

visual
Parkinsonian
greater
fluctuations

Front 45

The course of dementia due to Lewy Body Disease is the same as that for Alzheimer's.

TRUE or FALSE

Back 45

FALSE

The onset is similar; however, Lewy Body Disease has a more rapid course

Front 46

Because patients with dementia due to Lewy Body Disease have Parkinsonian movement disorders, they are responsive to l-Dopa.

TRUE or FALSE

Back 46

FALSE

They are not responsive to l-Dopa, but they are overly sensitive to dopamine blocking agents

Front 47

Where are Lewy Bodies found in Diffuse Lewy Body Dementia?

Back 47

In the cortex, rather than confined to the basal ganglia as they are in Parkinson's patients.

Front 48

Creutzfeldt-Jacob's Disease is a common transmitable virus that has a prevalance of 1 per 50,000.

TRUE or FALSE

Back 48

FALSE

Creutzfeldt-Jacob Disease is very rare, occuring in only 1 per 1 million people.

Front 49

What is a prion?

Back 49

It stands for proteinaceous infectious particle.

It is an infectious agent that is made only from protein.

Front 50

Typical presenting features of Creutzfeldt-Jacob's Disease include:

a)myoclunus, visual distortions
b) ataxia
c) rapidly progressive dementia, an exagerated startle response
d) hallucinations
e) a, b, and d
f) a and c
g) a, b, and c
h) all of the above
i) none of the above

Back 50

g) a, b, and c

Front 51

Creutzfeldt-Jacob's Disease can only be contracted by being exposed to infectious material.

TRUE or FALSE

Back 51

FALSE

It can be inherited also.

Front 52

The course of Creutzfeldt-Jacob's Disease is very slow, that is why it has been known to be a "slow virus."

TRUE or FALSE

Back 52

FALSE

The course is actually very rapid, 6-12 months in length.

The reason it is known as a "slow virus" is because they can have an incubation period of 2-25 years.

Front 53

What is myoclonus?

Back 53

One or a series of shock-like contractions of a group of muscles, of variable regularity, synchrony, and symmetry, generally due to a central nervous system lesion.

Front 54

What are prion-related dementias?

Back 54

1. Creutzfeldt-Jacob Disease
2. Kuru
3. Fatal Familial Insomnia
4. Gerstmann-Straussler-Scheinker syndrome

Front 55

What are the typical features of Frontal Lobe Dementia?

Back 55

1. Changes in personality
2. Breakdown in social conduct
3. Loss of social awareness and emotional empathy
4. Disinhibition (the major finding)
5. Impulsivity
6. Unconcern
7. Changes in eating conduct (hyperphasia)
8. Stereotyped and perseverative behavior

Front 56

Dementia is not typically seen in Huntington's Chorea.

TRUE or FALSE

Back 56

FALSE

Dementia is a uniform part of the disease and may be the initial symptom preceding the appearance of chorea or other abnormalities.

Front 57

What neuranatomical areas are typically affected in Huntington's Chorea?

a) caudate and putamen
b) frontal cortex and corpus callosum
c) basal ganglia and temporal lobes
d) thalamus
e) a, b, c
f) b, c, d
g) a, b, d

Back 57

g) a, b, d

A degenerative loss of neurons is seen in these areas due to a genetic abnormality

Front 58

What is the prevalence of Huntington's Chorea?

a) 1/1,000,000
b) 1/100,000
c) 5/500,000
d) 5/100,000

Back 58

d) 5/100,000

Front 59

What chromosome has been associated with Huntington's Chorea?

a) 1
b) 4
c) 21
d) 12

Back 59

b) 4

Front 60

Children manifesting signs of Huntington's Chorea will demonstrate cognitive or affective symptoms first.

TRUE or FALSE

Back 60

FALSE

Children will typically show parkinsonian symptoms first; while later onset will typically manifest the cognitive or affective symptoms first.

Front 61

Neuropsychological testing may be a helpful predictor for individuals at-risk for developing Huntington's Chorea.

TRUE or FALSE

Back 61

TRUE

At-risk groups have been shown to perform poorly on frontal lobe tests.

Front 62

Dementia features of Huntington's Chorea DOES NOT include:

a) apraxia
b) attention/concentration
c) executive abilities
d) memory
e) aphasia
f) agnosia
g) a, f
h) b, c
i) a, e, f
j) all of the above
k) none of the above

Back 62

i) a, e, f

Front 63

What are the characteristics of motor abnormality seen in Huntington's Chorea?

Back 63

1. Movement NEVER involves single muscles but include whole limbs or parts of a limb.
2. Movements are irregular and follow no sense of pattern
3. Eventually the movements become uncontrollable and affect the head, face, trunk, and limbs, impeding all voluntary movements including speech and swallowing.

Front 64

Other symptoms of Huntington's Chorea include:

Back 64

1. Slow voluntary movements
2. Impaired saccades/occular disturbance
3. Gait abnormalities
4. Psychiatric disturbance (~50%)
5. Language is affected later in the disease, but it is hard to test because of dysarthria.

Front 65

What should be considered if someone is demonstrating symtpoms of Huntington's Chorea, but does not have a positive family history for it?

Back 65

Wilson's Disease

Front 66

Huntington's Chorea is caused by a hyperactive norepinephrine system.

TRUE or FALSE

Back 66

FALSE

It is thought that neurotransmitters which normally inhibit the dopamine pathways (GABA and norepinephrine) die during the course of the disease, thereby creating a hyperactive dopamine system, which leads to the chorea.

Front 67

What causes Parkinson's Disease?

Back 67

The degeneration of the substantia nigra, which produces the dopamine source for the basal ganglia. As the substantia nigra degenerates, it stops producing dopamine.

Front 68

Parkinson's patients show a decrease of brain dopamine of over:

a) 25%
b) 50%
c) 75%
d) 90%

Back 68

d) 90%

Front 69

Parkinson's Disease is the most common extrapyramidal disorder affecting:

a) 1/100 over age 65
b) 1/1,000 over age 65
c) 1/10,000 over age 65
d) 1/100,000 over age 65

Back 69

a) 1/100 over age 65

Front 70

Parkinson's Disease is an inherited disorder.

TRUE or FALSE

Back 70

FALSE

Front 71

Clinically diagnosable dementia occurs in approximately what % of Parkinson's patients?

Back 71

20-60%

Front 72

What are the features of Parkinson's Disease?

Back 72

1. Movement disorder
2. Bradyphrenia (mental slowing)
3. Memory problems
4. Executive problems
5. Attentional difficulties
6. Visuospatial difficulties
7. Depression (~30%)

Front 73

Are cortical disturbances like aphasia and apraxia typically seen in Parkinson's patients?

Back 73

No

Front 74

What is the classic triad of Parkinson's Disease?

a) resting tremor, gait disturbance, posture disturbance
b) gait distubance, posture disturbance, bradykinesia
c) resting tremor, cogwheel rigidity, bradykinesia
d) resting tremor, cogwheel rigitiy, gait disturbance

Back 74

c) resting tremor, cogwheel rigidity, bradykinesia

Disturbance of posture is also quite frequent

Front 75

What are the POSITIVE SYMPTOMS of Parkinson's Disease?

Back 75

Actions which are not seen in normals

1. Resting tremor - "pill rolling"; are frequently ASYMMETRICAL; stops during voluntary movements or during sleep
2. Muscular rigidity - cogwheel effect, muscles allow movement for a short distance and then resist movement again
3. Involuntary movements - akathisia, meaning inability to remain in one posture

Front 76

What are the NEGATIVE SYMPTOMS of Parkinson's Disease?

Back 76

Inability to engage in behaviors that normals can

1. Posture disorder - inability or difficulty in maintaining a part of the body in normal position (e.g., head drooping)
2. Righting disorders - difficulty in standing from a sitting position
3. Locomotion disorders - difficulty initiating stepping, they shuffle; often once start walking, take faster and faster steps until they end up running (festinating gait)
4. Speech disturbance - difficulties in the physical production of sound
5. Akinesia - poverty or slowness of movement (e.g., blank facial expression, lack of blinking, etc.)
6. Aphagia - difficulty chewing/swallowing.

Front 77

Medications help reduce motor and cognitive symptoms in Parkinson's Disease.

TRUE or FALSE

Back 77

FALSE

Medications can help with motor symptoms, but do not improve cognitive deficits; in fact, some patients may become confused with the medication

Front 78

Surgical interventions can help which aspects of Parkinson's Disease?

Back 78

1. Pallidotomy - dyskinesia (abnormality in performing voluntary muscle movements)

2. Thallotomy - tremor

Front 79

What is the name of the dementia that results from the degeneration of multiple structures including the superior colliculus, red nucleus, dentate nucleus, subthalamic nucleus, and globus pallidus?

a) Parkinson's
b) Alzheimer's
c) Wilson's
d) Progressive Supranuclear Palsy

Back 79

d) Progressive Supranuclear Palsy

Front 80

Progressive Supranuclear Palsy is earmarked by parkinsonism symptoms, swallowing difficulty, and supranuclear opthalmoplegia.

TRUE or FALSE

Back 80

TRUE

Front 81

Some of the more common features of Progressive Supranuclear Palsy are?

Back 81

1. Gaze paresis - loss of downward gaze, leading to frequent falls and "dirty tie" sign
2. Axial rigidity - trunk/neck rigidity producing erect or hypererect rather than stooped posture.
3. Bradykinesia
4. Pseudobulbar palsy - masked face, increased jaw and facial jerks, exaggerated palatal and pharyngeal reflexes, dysphagia, drooling, emotional lability
5. Gait disorder - falls and akinesia (motionlessness attributable to a temporary paralysis) can also be present
6. "Wide-eyed stare"

Front 82

What is the peak onset for Wilson's Disease?

Back 82

Teens - 20s

Front 83

What is the cause of Wilson's Disease?

Back 83

Abnormalities in copper metabolism

Front 84

Wilson's Disease is NOT inheritable.

TRUE or FALSE

Back 84

FALSE

Wilson's Disease is a disorder of an autosomal recessive gene on chromosome 13

Front 85

Are the effects of Wilson's Disease reversible?

Back 85

Yes - if caught early treatment which includes restriction of copper intake and enhancing copper elimination can reverse haptic, renal, and cerebral dysfunction.

Front 86

Wilson's Disease includes all but which of the following:

a) Parkinsonina symptoms
b) Psychosis
c) Dementia
d) Mood changes
e) Wandering tendencies
f) Kayser-Fleischer rings

Back 86

e) Wandering tendencies

Front 87

Do Kayser-Fleischer rings show up on all patients with Wilson's Disease?

Back 87

No - these copper corneal rings, while pathognomonic, may not be seen in every patient

Front 88

What is Hallervorden-Spatz Disease?

Back 88

A rare inherited and progressive illness with late childhood or early adolescent onset.

It is characterized by dementia with spasticity and rigidity, dystonia or chorea.

Front 89

What causes Hallervorden-Spatz Disease?

Back 89

An accumulation of iron in the brain, for which there is no current cure.

Front 90

What is Sydenham's Chorea?

Back 90

a.k.a. - "Saint Vitus Dance"

A disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands.

Front 91

What is Sydenham's Chorea typically associated with?

Back 91

Rheumatic fever

Front 92

Sydenham's Chorea typically affects adults between the ages of 30 and 65.

TRUE or FALSE

Back 92

FALSE

Most patients are children under the age of 18

Front 93

Sydenham's Chorea is progressive and incurable.

TRUE or FALSE

Back 93

FALSE

50% of Sydenham's Chorea patients recover spontaneously after 2-6 months, while mild to moderate chorea or other motor symptoms can persist for up to and over 2 years.

Front 94

Sydenham's Chorea is associated with all but which of the following:

a) Tics
b) Tourette's
c) ADHD
d) Psychosis
e) OCD
f) Lupus

Back 94

d) Psychosis

Front 95

What is dementia pugilistica?

Back 95

A form of head trauma-related dementia seen mainly in boxers who suffer recurrent head injuries, in which there is parkinsonism and cognitive decline.

aka - Punch Drunk Syndrome

Front 96

What kinds of neurological changes are seen in dementia pugilistica?

Back 96

1. White matter changes
2. Focal contusions
3. Cerebral atrophy in porportion to the number of fights (especially the corpus callosum and cerebrum)
4. Hydrocephalous
5. Depigmented substantia nigra
6. Neurofribrillary tangles
7. Amyloid plaques